Article ; Online: Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.
Rheumatology (Oxford, England)
2022 Volume 62, Issue 5, Page(s) 1877–1886
Abstract: Objectives: To establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD).: Methods: A conceptual framework for subclinical, clinical and progressive ILD was provided to 83 ... ...
| Abstract | Objectives: To establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD). Methods: A conceptual framework for subclinical, clinical and progressive ILD was provided to 83 experts, asking them to use the framework and classify actual SSc-ILD patients. Each patient profile was designed to be classified by at least four experts in terms of severity and risk of progression at baseline; progression was based on 1-year follow-up data. A consensus was reached if ≥75% of experts agreed. Experts provided information on which items were important in determining classification. Results: Forty-four experts (53%) completed the survey. Consensus was achieved on the dimensions of severity (75%, 60 of 80 profiles), risk of progression (71%, 57 of 80 profiles) and progressive ILD (60%, 24 of 40 profiles). For profiles achieving consensus, most were classified as clinical ILD (92%), low risk (54%) and stable (71%). Severity and disease progression overlapped in terms of framework items that were most influential in classifying patients (forced vital capacity, extent of lung involvement on high resolution chest CT [HRCT]); risk of progression was influenced primarily by disease duration. Conclusions: Using our proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time. Experts rely on similar items when classifying disease severity and progression: a combination of spirometry and gas exchange and quantitative HRCT. |
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| MeSH term(s) | Humans ; Lung Diseases, Interstitial/complications ; Scleroderma, Systemic/complications ; Vital Capacity ; Tomography, X-Ray Computed/methods ; Severity of Illness Index ; Lung | ||||||||||
| Language | English | ||||||||||
| Publishing date | 2022-09-29 | ||||||||||
| Publishing country | England | ||||||||||
| Document type | Journal Article ; Research Support, N.I.H., Extramural | ||||||||||
| ZDB-ID | 1464822-2 | ||||||||||
| ISSN | 1462-0332 ; 1462-0324 | ||||||||||
| ISSN (online) | 1462-0332 | ||||||||||
| ISSN | 1462-0324 | ||||||||||
| DOI | 10.1093/rheumatology/keac557 | ||||||||||
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| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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